| umls-concept:C0751690 | skos:definition | NCI: A type of soft tissue sarcoma that develops in cells that form a protective sheath (covering) around peripheral nerves, which are nerves that are outside of the central nervous system (brain and spinal cord).,NCI: A malignant neoplasm, originating from the sheaths of the peripheral nerve.,NCI: An uncommon, highly aggressive malignant tumor, arising from the peripheral nerves and affecting mostly adults in their third to sixth decades of life. It usually occurs in medium-sized and large nerves of the buttock, thigh, upper arm, or the paraspinal region. It may be associated with neurofibromatosis 1 (NF1).,NCI: Any malignant tumor arising from a peripheral nerve or showing nerve sheath differentiation, with the exception of tumors originating from the epineurium or the peripheral nerve vasculature. Approximately 50% of cases are associated with neurofibromatosis 1. | lld:umls |
